Medicina: Ematologia: Talassemie e trapianto del midollo osseo
| English |
| hepatosplenomegaly |
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| Attestation |
3
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| Part of speech |
Noun
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| Grammatical label |
Countable
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| Definition |
Ineffective erythropoiesis is responsible for many of the undesirable manifestations of the disease in its untreated state. Hepatosplenomegaly, due to extramedullary hematopoiesis, and bony malformations (including the facial deformities resulting in the so-called ´thalassemic facies´) are among these manifestations. However, another main histopathological finding in these organs is gross hyperplasia of reticulo-endothelial cells (macrophages, Kuppfer cells, etc.) containing red cells and their breakdown products.
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| Definition source |
Mohamed, N. & Jackson, N. (1998). ‘Severe thalassemia intermedia: clinical problems in the absence of hypertransfusion’. Blood Reviews 12(3):163-70. (RISCEN83)
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| Context |
The two probands were a 13-year old boy (proband 1) and a 14-year old girl (proband 2), who presented with a history of easy fatiguability, excessive sweating and somnolence for years. Though with normal growth and development, they both had moderate anemia, jaundice, hepatosplenomegaly and some facial bone alteration.
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| Context source |
Li, Z., et al. (2001). ‘Two thalassemia intermedia patients with delta beta/beta-thalassemia and a deletional type alpha-thalassemia’. Haematologica 86(1):108. (RISCEN30)
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| Subject field |
Haemopoiesis
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| Sub-field (level 1) |
Thalassemias
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| Partitive concept |
Epatomegaly, splenomegaly, hypersplenism
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| Related concept |
Hepatopathy, splenectomy, iron-chelation therapy, iron-chelating agent
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| it |
Epatosplenomegalia
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| Reliability code |
3
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